Moyamoya Disease

Also known as: "Puff of smoke", moyamoya syndrome

What is Moyamoya disease?

Moyamoya disease is a rare disease, frequently seen in children, that causes the blood vessels in the neck to narrow. This causes the blood flow to the brain to slow which can lead to blood clots, starving the child's brain of the oxygen it needs to function. The reduction in blood flow causes a tangled network of new blood vessels to grow which on X-ray studies looks like a "puff of smoke", (this is what "moyamoya" means in Japanese).
 

What causes Moyamoya disease?

The cause of the disease is unknown, however, in 10% of affected patients it appears to be familial (genetic). It is also more common in people from Asian countries, (though it does occur in Western nations, as well). Sometimes Moyamoya disease is associated with a number of other disease states.
 

What are the symptoms of Moyamoya disease?

Unfortunately, moyamoya disease typically doesn’t cause symptoms until a transient ischemic attack (TIA), or mini-stroke, occurs. This can cause headaches, dizziness, seizures, weakness or paralysis of a portion of the body.


What are moyamoya disease care options?

The disease usually requires surgery to restore the flow of blood to the brain. This can successfully treat moyamoya disease in many people, particularly children, though clinical  results will depend on whether a stroke has occurred.

Reviewed by: Jack Wolfsdorf, MD, FAAP

This page was last updated on: 10/31/2017 11:51:38 AM

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